1: Arch Dis Child Fetal Neonatal Ed 1995 Nov;73(3):F178-80

Liver transplantation for neonatal haemochromatosis

Muiesan P, Rela M, Kane P, Dawan A, Baker A, Ball C, Mowat AP, Williams R,
Heaton ND
Liver Transplant Surgical Service, King's College Hospital, Camberwell, London

Abstract
Two cases of neonatal haemochromatosis, a rare and often fatal metabolic
disorder, presenting with acute liver failure, are reported. Both presented in
the first week of life with hypoglycaemia, jaundice, and coagulopathy, with
rapid deterioration of liver function. Both received a transplantation using
reduced liver grafts. One child was well 18 months later. Few survivors have
been reported and despite the difficult perioperative management, liver
transplantation is the best treatment for neonatal haemochromatosis